Pregnant woman presents with blurry vision
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A 30-year-old woman initially presented to Tufts/New England Eye Center as a referral from an outside retina specialist.
The patient was first discovered to have a large pigmented choroidal lesion in her right eye on routine examination approximately 3 years before presentation. At that time, the patient denied any ocular symptoms including blurry vision, loss of vision, flashes or floaters. Her examination was reported to have been stable while being followed by the referring specialist. At Tufts, she was followed for another 2 years, during which she remained asymptomatic. However, she subsequently developed 1 month of blurry vision in her right eye and presented for evaluation.
Her ocular history was significant only for myopia (–2 D in both eyes). At the time of symptom onset, the patient reported she was 37 weeks pregnant. Her remaining medical, family and social histories were noncontributory. She had no known drug allergies and denied use of alcohol, tobacco or recreational drugs. A review of systems was otherwise negative.
Examination
Upon examination, visual acuity was 20/200 in the right eye (previously 20/20) and 20/20 in the left eye with correction. Pupils were equal and reactive bilaterally without relative afferent pupillary defect. Extraocular motility was full and painless bilaterally. Confrontation visual field and color vision testing were full in both eyes. IOPs were within normal limits bilaterally.
Anterior segment exam of the right and left eyes was unremarkable. Dilated fundus exam of both eyes demonstrated pink and healthy-appearing optic nerves with a 0.4 cup-to-disc ratio. At baseline, before symptom onset, a darkly pigmented choroidal lesion could be appreciated throughout the macula and peripapillary region, extending inferiorly in the right eye. Scattered drusen were present through the lesion. No orange pigment, subretinal fluid or subretinal hemorrhage was noted (Figure 1a). Fundus autofluorescence demonstrated hyperautofluorescence (Figure 1b) throughout the pigmented lesion, with B-scan of the right eye showing a flat choroid without thickening or hyperechogenicity (Figure 1c). The posterior pole of the left eye was unremarkable. When the patient was seen for follow-up after developing blurry vision, a repeat dilated exam showed an unchanged lesion size with a newly noted serous retinal detachment with shifting fluid inferiorly.
Baseline OCT of the macula demonstrated normal retinal contour with a compressed choroid (Figure 1d). Repeat OCT of the macula at the time of symptom onset demonstrated new subfoveal subretinal fluid (Figure 1e). Fluorescein angiography (FA) demonstrated early and late staining without frank leakage in the right eye (Figure 2a). Indocyanine green (ICG) angiography showed late pinpoint leakage just superior to the inferior arcade overlying the pigmented choroidal lesion (Figure 2b).
Source: Jonathan T. Caranfa, MD, PharmD, and Shilpa Desai, MD
What is your diagnosis?
Choroidal lesion
In this patient with long-term stability of a known pigmented choroidal lesion, physiologic changes during the third trimester of her pregnancy most likely contributed to the development of new subretinal fluid and serous retinal detachment. Nonetheless, other differential diagnoses must be considered.
When evaluating a pigmented choroidal lesion, the two most common diagnoses to consider are choroidal melanoma and choroidal nevus. Choroidal melanomas are commonly seen as raised lesions, whereas nevi tend to be flat in nature. To further aid in differentiating between a benign nevus and malignant melanoma, Shields and colleagues published a large retrospective analysis describing the factors predictive of choroidal nevus transformation into choroidal melanoma. In this paper, they developed the mnemonic “to find small ocular melanoma using helpful hints daily,” describing features such as thickness greater than 2 mm, fluid, symptoms (blurry vision, flashes, floaters, etc), orange pigment, margin less than 3 mm to disc, ultrasonographic hollowness, halo absence and drusen absence.
Other differential diagnoses for pigmented choroidal lesions include metastatic lesions, congenital hypertrophy/reactive hyperplasia of the retinal pigment epithelium (RPE) and choroidal melanocytosis.
When fluid is present in an otherwise stable pigmented lesion, one should certainly consider malignant transformation; however, cases of nevus leakage due to RPE remodeling, superimposed central serous chorioretinopathy (CSCR) and choroidal neovascular membrane formation have all been described in the literature. Physiologic changes during pregnancy may contribute to the development of subretinal fluid associated with existing choroidal nevi, although the exact mechanisms are unknown.
Workup and management
In patients presenting with a suspicious-appearing choroidal lesion, it is important to gather pertinent clinical history including risk factors such as smoking, personal or family history of melanoma or other cancers, and visual symptoms such as decreased visual acuity, visual field defects, floaters and/or photopsias. Dilated examination should be performed in both eyes to assess for symmetry/presence of additional lesions with special attention being paid to concerning features such as size, elevation, fluid and orange pigmentation within the lesion. Features such as drusen or depigmentation surrounding the pigmented portion (ie, halo) may suggest a benign lesion.
In addition to clinical examination, several diagnostic procedures can assist in making the diagnosis and guiding treatment. Serial fundus photography is imperative in tracking lesion size and identifying the development of suspicious features over time. A- and B-scan ultrasonography can assess for low to medium internal echogenicity, choroidal excavation and shadowing, all of which are concerning for choroidal melanoma. Moreover, ultrasound can measure lesion depth and size, which, similar to fundus photography, should be tracked over time. OCT imaging through either the macula or the lesion itself can detect subtle serous retinal detachments, and FA/ICG angiography is best used to identify high-risk features such as leakage or irregular vessels within and/or around the lesion.
Discussion
Uveal or choroidal melanoma is the most common intraocular malignancy in adult patients with a rate of approximately 5.1 cases per million. Uveal melanoma is most frequently found incidentally on routine dilated examination and tends to arise in white individuals between the fifth and sixth decade of life. In a patient with acute onset of new subretinal fluid in the setting of a known pigmented choroidal lesion, malignant transformation to melanoma is one concern. Biopsy can be considered if suspicion for malignant transformation is high, particularly if the underlying lesion has grown in size.
A diagnosis of CSCR should also be considered with the findings of new subretinal fluid and an area of pinpoint leakage on FA/ICG angiography. In a recent case-control study, Haimovici and colleagues found pregnancy (especially in the third trimester) to be the second most causative risk factor, following corticosteroid use, in the development of CSCR. Increased levels of progesterone and endogenous steroid hormone biosynthesis used in maintenance of the growing fetus are theorized to alter choroidal blood flow, leading to the RPE hyperpermeability seen in CSCR.
Multiple treatment options exist for patients with subretinal fluid associated with choroidal nevi. In patients with known choroidal neovascular membranes, anti-VEGF intravitreal injections have demonstrated efficacy in reducing subretinal fluid, improving visual acuity and resulting in long-term regression of these membranes. In patients without choroidal neovascularization, photodynamic therapy (PDT) has been utilized with documented success in reducing subretinal fluid.
The basis of PDT is centered around intravenous infjection of Visudyne (verteporfin, Bausch + Lomb), which preferentially accumulates within abnormal retinal vasculature. When verteporfin comes in contact with light, it becomes activated and reacts with oxygen to form oxygen free radicals. These free radicals cause localized endothelial tissue damage, activation of the clotting cascade and ultimately microvascular occlusion. Standard treatment protocol consists of a dose of 6 mg/m2 of verteporfin combined with a non-thermal red laser (689 nm) of 50 J/cm2 (fluence) over 83 seconds. Given concerns for retinal ischemia, RPE atrophy and fibrosis, some providers have adopted “safety-enhanced protocols,” which reduce the dose, energy and/or duration of laser treatment.
Clinical course continued
At the time of symptom onset, the patient’s lesion had been stable for more than 5 years, with a known possible precipitating factor (pregnancy) for her acute serous retinal detachment. A biopsy would not have been feasible given the lack of elevation of the lesion (generally 2 mm of elevation is required). Given these factors, and in light of her being in the third trimester of pregnancy, she elected for observation and to have a repeat evaluation 4 weeks after delivery to reassess the subretinal fluid. Examination at that time demonstrated an unchanged pigmented choroidal lesion, with improved subretinal fluid and stable visual acuity of 20/200 in the right eye.
The patient was followed closely on a monthly basis without significant improvement in subretinal fluid or visual acuity. After several months of observation, the patient elected to undergo PDT (half time, half fluence). At her 1-month follow-up, visual acuity remained unchanged with no improvement noted on clinical examination. After a long discussion, the patient elected to undergo a repeat trial of PDT at full time and full fluence, which will be scheduled in the coming months. She will continue to be monitored with serial exams.
- References:
- Chiang A, et al. Retina. 2012;doi:10.1097/IAE.0b013e31822092b7.
- García-Arumí J, et al. Retina. 2012;doi:10.1097/IAE.0b013e318232c366.
- Haimovici R, et al. Ophthalmology. 2004;doi:10.1016/j.ophtha.2003.09.024.
- Ju Y, et al. Bioact Mater. 2022;doi:10.1016/j.bioactmat.2022.01.028.
- Newman DK. Eye (Lond). 2016;doi:10.1038/eye.2015.251.
- Nkrumah G, et al. Ther Adv Ophthalmol. 2020;doi:10.1177/2515841420950846.
- Pointdujour-Lim R, et al. Retina. 2017;doi:10.1097/IAE.0000000000001202.
- Shields CL, et al. Arch Ophthalmol. 2009;doi:10.1001/archophthalmol.2009.151.
- For more information:
- Edited by Jonathan T. Caranfa, MD, PharmD, and Angell Shi, MD, of New England Eye Center, Tufts University School of Medicine. They can be reached at jcaranfa@tuftsmedicalcenter.org and ashi@tuftsmedicalcenter.org.
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